Ember's Journey: September 2008

When Ember was born we noticed a flat spot on the right side of her forehead. The pediatrician told us it was from her position in the womb, and that it would round out in a few weeks. When her 2 week checkup came around I asked the doctor if she would need one of those "head-shaper helmet thingies" because I was concerned about how her right eye seemed to be bugging out a little bit. She said we should wait a little longer for the head to round out, and referred us to an opthamologist to rule out glaucoma (increased eye pressure) as the cause of the difference in the eyes. Of course I was horrified at the idea of my 2-week-old having glaucoma, but the opthamologist did all the tests & told me that her eyes are perfectly fine - the apparent difference in the eyes is just because of the shape of Ember's forehead. So this past Monday Ember had her 2 month checkup and I brought up the issue again because the shape doesn't seem to be improving at all. This time I was referred to an orthotic specialist to have her evaluated for a helmet. The appointment was yesterday. He said that he couldn't give her a helmet because he believes she has Craniosynostosis, specifically Right Coronal Craniosynostosis. She has all of the distinct symptoms (forehead flattened on one side & bulging on the other) , including the more rare ones like crooked nose, one eye lower than the other, and uneven ears. Cranio can only be confirmed by a 3D CT scan, so we are waiting for an appointment to get the scan done.

Some Cranio info:

What is Craniosynostosis?

Craniosynostosis is a congenital anomaly characterized by the premature closure of one or more cranial sutures (the fibrous joints between the bones of the skull). The disorder results in an abnormal skull and head shape. Most cases are sporadic, although there are several genetic syndromes with Craniosynostosis. Disorders of the fibroblast growth factor receptor molecule have been recently found in some cases of hereditary Craniosynostosis.

What Are The Symptoms of Craniosynostosis?

Most children with Craniosynostosis do not have any symptoms; the head shape is abnormal, and the face may be malformed in certain cases.
Rarely, symptoms of increased pressure in the head such as headaches, decreased appetite, vomiting, or even developmental delays or mental retardation may occur.

How Does A Craniofacial Anomaly Occur?

Five separate bones make up the cranium of the head: the occipital bone (back portion of the skull); two parietal bones (on either side of the skull); and two frontal bones (front portion of the skull). The bones, which are in place by the 5th month of gestation, are like pieces of a puzzle, and are initially separated by sutures (or fibrous joints). The skull bones, coupled with the sutures, form a protective shield for the brain.
As the brain grows, the sutures allow the skull to expand rapidly. If for any reason one or more of the sutures closes too early, the skull grows in a different direction where the bones are not resisting growth, thereby resulting in skull deformities.

What Treatments Are Available?

Craniosynostosis surgery is commonly offered to correct the cosmetic deformity of the skull and face. Rarely surgery is necessary to reduce intracranial pressure and allow for brain growth; however, it is sometimes performed when multiple sutures are involved.
The timing and type of surgery is very much dependent on the type of synostosis and the preference of the surgeon. Most often, surgery for craniosynostosis is performed during the first several months of life.
Helmets or headband therapy may also be used as an adjunct to surgery for selected cases. Some neurosurgeons use headbands for occipital plagiocephaly. In this type of therapy, the child wears a customized, external helmet or headband for a prescribed period to direct proper skull growth.